Multiple neoplasms in an adolescent child associated with IgA deficiency

Abstract

A report is presented of an unusual occurrence of multiple primary malignant neoplasms in a young girl. Beginning at the age of 10 she had multiple adenomatous polyps removed from the colon over a 2-year period. One of the polyps had a malignant transformation. During the ensuing 8 years, she developed malignant thymoma, squamous cell carcinoma of the scalp, adenocarcinoma of the colon, which recurred, and choroid tumor of the eye. She died at the age of 20 with malignant astrocytoma of the cerebrum. A brother died at the age of 16 because of lymphoma, lymphocytic, well differentiated, with invasion of the spinal column and meninges. The girl had a serum IgA less than 20% of normal level and her brother had total absence of IgA.